Rabbit anti-Human Aconitase 2 Polyclonal Antibody | anti-ACO2 antibody

Anti-Aconitase 2 Antibody, Rabbit Polyclonal

Cat. Num. AAA8104424

• Gene Names: ACO2; ICRD; ACONM
• Reactivity: Human
• Applications: Western Blot, ELISA, Immunohistochemistry, Immunoprecipitation
• Purity: Protein A & Antigen Affinity
• Synonyms: Aconitase 2; Polyclonal Antibody; Anti-Aconitase 2 Antibody; Rabbit Polyclonal; ACO2/Aconitase 2 Antibody; Rabbit PAb; Antigen Affinity Purified; aconitase 2; mitochondrial; Anti-ACONM Antibody; Anti-ICRD Antibody; anti-ACO2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human ACO2/Aconitase 2
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-ACO2 antibody: Western Blot (WB), ELISA (EIA), Immunohistochemistry-Paraffin (IHC-P), Immunoprecipitation (IP)
• Application Notes: WB: 1:500-1:2000; ELISA: 1:5000-1:10,000; IHC-P: 1:500-1:2000; IP: 1-4uL/mg of lysate
• Immunogen: Recombinant Human ACO2/Aconitase 2 Protein
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with purified, recombinant Human ACO2/Aconitase 2 (rh ACO2/Aconitase 2). ACO2/Aconitase 2 specific IgG was purified by Human ACO2/Aconitase 2 affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunochemical staining of human ACO2 in human skeletal muscle with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).)

Immunohistochemistry (IHC)

(Immunochemical staining of human ACO2 in human kidney with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).)

Immunoprecipitation (IP)

(ACO2 was immunoprecipitated using:Lane A:0.5 mg K562 Whole Cell LysateLane B:0.5 mg Hela Whole Cell LysateLane C:0.5 mg Jurkat Whole Cell Lysate4 uL anti-ACO2 rabbit polyclonal antibody and 15 ul of 50 % Protein G agarose.Primary antibody:Anti-ACO2 rabbit polyclonal antibody,at 1:100 dilution Secondary antibody:Dylight 800-labeled antibody to rabbit IgG (H+L), at 1:5000 dilution Developed using the odssey technique.Performed under reducing conditions.Predicted band size: 85 kDaObserved band size: 85 kDa)

Western Blot (WB)

(Anti-ACO2 rabbit polyclonal antibody at 1:500 dilution.Lane A: Rat heart tissue lysateLane B: Rat Brain tissue lysateLane C: Mouse Brain tissue lysateLane D: Jurkat Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti- Rabbit  IgG H&L (Dylight 800)  at 1/10000 dilution.Developed using the Odyssey technique. Performed under reducing conditions.Predicted band size:85 kDaObserved band size:92 kDa(We are unsure as to the identity of these extra bands.))

Related Product Information for anti-ACO2 antibody

A homozygous missense mutation was identified in the ACO2 gene (c.1240T>G p.Phe414Val) that segregated with HSP complicated by intellectual disability and microcephaly. Lymphoblastoid cell lines of homozygous carrier patients revealed significantly decreased activity of the mitochondrial aconitase enzyme and defective mitochondrial respiration. ACO2 encodes mitochondrial aconitase, an essential enzyme in the Krebs cycle. Recessive mutations in this gene have been previously associated with cerebellar ataxia. We found homozygous or compound heterozygous missense and frameshift mutations in the gene encoding mitochondrial aconitase (ACO2), a tricarboxylic acid cycle enzyme, catalysing interconversion of citrate into isocitrate. Unlike wild type ACO2, all mutant ACO2 proteins failed to complement the respiratory growth of a yeast aco1-deletion strain. The study shows that autosomal recessive ACO2 mutations can cause either isolated or syndromic optic neuropathy. This observation identifies ACO2 as the second gene responsible for non-syndromic autosomal recessive optic neuropathies and provides evidence for a genetic overlap between isolated and syndromic forms, giving further support to the view that optic atrophy is a hallmark of defective mitochondrial energy supply.

References

Robbins AH, et al. (1989) The structure of aconitase. Proteins. 5 (4): 289-312.Lauble H, et al. (1992) Crystal structures of aconitase with isocitrate and nitroisocitrate bound. Biochemistry. 31 (10): 2735-48.Robbins AH, et al. (1989) Structure of activated aconitase: formation of the 4Fe-4S cluster in the crystal. Proc Natl Acad Sci. 86 (10): 3639-43.

NCBI and Uniprot Product Information

• NCBI GI #: 6686275
• NCBI GeneID: 50
• UniProt Accession #: Q99798; O75809; Q5JZ41; Q6FHX0; Q8TAQ6
• Molecular Weight: 780
• NCBI Official Full Name: Aconitate hydratase, mitochondrial
• NCBI Official Synonym Full Names: aconitase 2, mitochondrial
• NCBI Official Symbol: ACO2
• NCBI Official Synonym Symbols: ICRD; ACONM
• NCBI Protein Information: aconitate hydratase, mitochondrial; citrate hydro-lyase
• UniProt Protein Name: Aconitate hydratase, mitochondrial
• Protein Family: Aconitate hydratase
• UniProt Gene Name: ACO2
• UniProt Synonym Gene Names: Aconitase
• UniProt Entry Name: ACON_HUMAN

NCBI Description

The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq, Jul 2008]

Uniprot Description

ACO2: Catalyzes the isomerization of citrate to isocitrate via cis-aconitate. Monomer. Belongs to the aconitase/IPM isomerase family.

Protein type: Lyase; EC 4.2.1.3; Carbohydrate Metabolism - glyoxylate and dicarboxylate; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial

Chromosomal Location of Human Ortholog: 22q13.2

Cellular Component: mitochondrion; mitochondrial matrix; nucleus

Molecular Function: iron ion binding; 4 iron, 4 sulfur cluster binding; aconitate hydratase activity; 3 iron, 4 sulfur cluster binding

Biological Process: isocitrate metabolic process; cellular metabolic process; generation of precursor metabolites and energy; tricarboxylic acid cycle; citrate metabolic process

Disease: Infantile Cerebellar-retinal Degeneration; Optic Atrophy 8